521 research outputs found

    The Learner’s Mirror. Designing a User Modelling Component in Adaptive Hypermedia Educational Systems

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    Thermal conductivity of amorphous polymers and its dependence on molecular weight

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    Thermal conductivity is an important transport property governing the performance of polymers in non-isothermal conditions. Nevertheless, its dependence on molecular weight M has not been the subject of as much attention as other properties of polymeric materials. We determine the thermal conductivity of polystyrene and polyisobutylene for a wide range of molecular weight by measuring the density, heat capacity and thermal diffusivity. Using coarse-graining and reverse mapping methods, we were able to produce molecular melts to study the thermal conductivity of polystyrene using molecular dynamics simulations over a similar range of molecular weight. We find satisfactory agreement between the experimental and simulation results. However, all of our results show that thermal conductivity depends only slightly on molecular weight up the entanglement limit and it is independent thereafter. Our results put into question the few previous experimental studies on this topic by showing that the previously accepted proportionality to M\sqrt{M} does not hold. Our findings could have significant implications for the understanding of complex phenomena such as anisotropic thermal conductivity in polymers subjected to flow.Marie Skłodowska-Curie IF MTCIATTP 750985

    Fine-time energetic electron behavior observed by Cluster/RAPID in the magnetotail associated with X-line formation and subsequent current disruption

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    Energetic electrons with 90deg pitch angle have been observed in the magnetotail at ~19 <i>R<sub>E</sub></i> near local midnight during the recovery phase of a substorm event on 27 August 2001 (Baker et al., 2002). Based on auroral images Baker et al. (2002) placed the substorm expansion phase between ~04:06:16 and ~04:08:19 UT. The electron enhancements perpendicular to the ambient magnetic field occurred while the Cluster spacecraft were on closed field lines in the central plasma sheet approaching the neutral sheet. Magnetic field and energetic particle measurements have been employed from a number of satellites, in order to determine the source and the subsequent appearance of these electrons at the Cluster location. It is found that ~7.5 min after an X-line formation observed by Cluster (Baker et al., 2002) a current disruption event took place inside geosynchronous orbit and subsequently expanded both in local time and tailward, giving rise to field-aligned currents and the formation of a current wedge. A synthesis of tail reconnection and the cross-tail current disruption scenario is proposed for the substorm global initiation process: When a fast flow with northward magnetic field, produced by magnetic reconnection in the midtail, abruptly decelerates at the inner edge of the plasma sheet, it compresses the plasma populations earthward of the front, altering dynamically the B<sub>z</sub> magnetic component in the current sheet. This provides the necessary and sufficient conditions for the kinetic cross-field streaming/current (KCSI/CFCI) instability (Lui et al., 1990, 1991) to initiate. As soon as the ionospheric conductance increases over a threshold level, the auroral electrojet is greatly intensified (see Fig. 2 in Baker et al., 2002), which leads to the formation of the substorm current wedge and dipolarization of the magnetic field. This substorm scenario combines the near-Earth neutral line and the current disruption for the initiation of substorms, at least during steady southward IMF. One can conclude the following: The observations suggest that the anisotropic electron increases observed by Cluster are not related to an acceleration mechanism associated with the X-line formation in the midtail, but rather these particles are generated in the dusk magnetospheric sector due to the longitudinal and tailward expansion of a current disruption region and subsequently observed at the Cluster location with no apparent energy dispersion.<br><br> <b>Keywords.</b> Magnetospheric physics (Magnetotail; Plasma convection; Storms and substorms

    Inter-familial and intra-familial phenotypic variability in three Sicilian families with Anderson-Fabry disease.

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    Abstract BACKGROUND: Anderson-Fabry disease (AFD) is an inborn lysosomal enzymopathy resulting from the deficient or absent activity of the lysosomal exogalactohydrolase, α-galactosidase A. This deficiency, results in the altered metabolism of glycosphingolipids which leads to their accumulation in lysosomes, thus to cellular and vascular dysfunction. To date, numerous mutations (according to recent data more than 1000 mutations) have been reported in the GLA intronic and exonic mutations. Traditionally, clinical manifestations are more severe in affected hemizygous males than in females. Nevertheless, recent studies have described severe organ dysfunction in women. THE AIM OF THE STUDY: This study reports clinical, biochemical, and molecular findings of the members of three Sicilian families. The clinical history of these patients highlights a remarkable interfamilial and intrafamilial phenotypic variability which characterizes Fabry disease relative to target organs and severity of clinical manifestations. DISCUSSION: Our findings, in agreement with previous data, report a little genotype-phenotype correlation for the disease, suggesting that the wide phenotypic variability of Anderson-Fabry disease is not completely ascribable to different gene mutations but other factors and mechanisms seem to be involved in the pathogenesis and clinical expression of the disease. Moreover, this study emphasies the importance of pedigree analysis in the family of each proband for identifying other possibly affected relatives
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